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MSK 2 > Rheum D/O 4 > Flashcards

Rheum D/O 4 Flashcards

1
Q

triple symptoms of Bahcet’s dz

A

recurrent uveitis
oral aphthous ulcers
genital ulcers

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2
Q

where is Bahcet’s dz MC

A

ancient Silk Road

easter EU to the Mediterranean

Turkish, Chinese, Japanese, Korean, Saudi Arabian

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3
Q

Bahcet’s dz patho

A

infectious pathogen triggers a hyperactive immune response in genetically susceptible person

possible heat shock proteins driving the reaction

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4
Q

proposed infectious agents of Bahcet’s dz

A 
herpes simplex
Strep 
Staph
H. Pylori 
E. coli
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5
Q

Bahcet’s dz levels of immunity affected

A

B and T cells
autoantibody production
activation of coagulation cascade

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6
Q

vessels Bahcet’s dz

A

vasculitis lesions of small, medium, and larger arteries and veins

VESSELS of ALL SIZES

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7
Q

epidemiology Bahcet’s dz

A

25-35 yrs

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8
Q

Bahcet’s dz spares

A

liver
kidneys
heart

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9
Q

clinical manifestations

A
  1. oral ulcers
  2. genital ulcers
  3. cutaneous lesions
  4. ocular disease
  5. neurologic dz
  6. arterial dz
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10
Q

Bahcet’s dz oral ulcers

A

canker sores

more extensive than typical

multiple, painful. limit eating

heal 1-3 weeks+

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11
Q

most specific sign for Bahcet’s dz

A

genital ulcers

painful, heal with scarring, most often on scrotum/vulva

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12
Q

cutaneous lesions (list)

A 
Acneiform
pathergy
dermaographism
erythema nodosum 
pyoderma hangrenosum
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13
Q

acneiform Bahcet’s dz

A

arthritis, looks like regular acne

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14
Q

pathergy Bahcet’s dz

A

formation of papule 48 hrs after skin prick

cutaneous reaction following skin stimulation

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15
Q

dermographism

A

light scratching produces pathology

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16
Q

erythema nodosum

A

medium vessel vasculitis that ULCERATES

17
Q

Bahcet’s dz making diagnosis

A

3 episodes of oral herpetiform or apthous ulceration in 12 months (observed or reported)

2 recurrent/painful genital ulcers and ophthalmic lesions

and
2 skin lesions or positive potherb skin testing

18
Q

pharm tx Bahcet’s dz

A 
gdlucocritcoids 
colchicine (mucocutaneous dz)
azathiprine 
severe immunosuppressive agents 
TNF Inhibitors
19
Q

polymyositis

A

idiopathic autoimmune inlammatory myopathy

  1. symmetrical, proximal muscle weakness
  2. elevated CK levels
  3. characteristic EMG and muscle bx findings
20
Q

dermatomyositis is different from polymyositis

A

due to associated rash

21
Q

dermatomyositis polymyositis epidemiology

specific agents

A

MC in African American

2x as common in women

dermatomyositis: children 5-14, adults 50+
polymyositis: adults, 45-60s

22
Q

etiology polymyositis

A

altered CELLULAR immunity

T cell mediated cytotoxic process against Ag on myocytes

23
Q

associated dz polymyositis

A

MC with other autoimmune dz

24
Q

dermatomyositis etiology

A

altered HUMERAL immunity

directed against muscle capillaries and small arteriole

complement activation = mircoinfarction

25
Q

dermatomyositis polymyositis association

A 
underlying malignancy 
infection 
medicaitons 
rheumatology disease
HLA subtypes
26
Q

infection of dermatomyositis polymyositis

A

HIV
coxsackievirus
HBV
influenza

27
Q

dermatomyositis polymyositis presentation

A

symmetrical and proximal painless myopathy

weakness of large muscles of hip and shoulder girdles develop over 3 - 6 months

weak neck muscles of neck flexion = difficult to hold head up

sensation and DTRs preserved

dysphagia

constitutional symptoms

interstitial lung disease

28
Q

dermatomyositis pathognomonic

A

heliotrope rash

29
Q

dermatomyositis polymyositis work up

A
  1. CPK (elevated 5-50x)
  2. EMG abnormal
  3. muscle biopsy
  4. malignancy screening
30
Q

dermatomyositis polymyositis tx (direct dz)

A

initial tx corticosteroids (prednisone) 1mg/kg/day x6-8 weeks and tapered

typically responds well, but if not give cytotoxic

vitamin D and Ca for osteoporosis

31
Q

dermatomyositis polymyositis CA

A

incidence of lung, bladder, non-Hodgkin lymphoma are all increased in 1st year after diagnosis

32
Q

prognosis dermatomyositis polymyositis

A

mortality is associated to malignancy and pulmonary complication

5 yr survival >80%

33
Q

inclusion body myositis

A

idiopathic autoimmune inflammatory myopathy

more common in men, >50

proximal and distal muscles affected

MSK 2 (10 decks)

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